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Journal of Pediatric Urology Aug 2021Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, a variable degree of urinary tract involvement and cryptorchidism. Most cases of PBS... (Review)
Review
Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, a variable degree of urinary tract involvement and cryptorchidism. Most cases of PBS are sporadic and have a normal karyotype, with 95% patients being male. In the last decade, mutations in known genes that regulate embryonic genitourinary myogenesis have been identified and with increasing knowledge of these critical genes involved in bladder maldevelopment, advances can be made in genetic counseling. A multidisciplinary approach is necessary and individualization of care is recommended according to phenotypic severity. Some patients require abdominal and urinary tract reconstruction while others require as little as bilateral orchiopexies. Major treatment objectives are: preservation of renal function and upper urinary tract; polyuria management; adequate bladder emptying; improvement of corporal image and quality of life; preservation of fertility and adequate sexual function. Long-term surveillance of the urinary tract is essential up to adulthood, because functional dynamics can change over time.
Topics: Adult; Cryptorchidism; Humans; Male; Orchiopexy; Prune Belly Syndrome; Quality of Life; Urinary Tract
PubMed: 34016542
DOI: 10.1016/j.jpurol.2021.04.010 -
Ginekologia Polska 2018Diastasis recti abdominis is a condition in which both rectus abdominis muscles disintegrate to the sides, this being ac-companied by the extension of the linea alba... (Review)
Review
Diastasis recti abdominis is a condition in which both rectus abdominis muscles disintegrate to the sides, this being ac-companied by the extension of the linea alba tissue and bulging of the abdominal wall. DRA may result in the herniation of the abdominal viscera, but it is not a hernia per se. DRA is common in the female population during pregnancy and in the postpartum period. There is a scant knowledge on the prevalence, risk factors, prevention or management of the abovemen-tioned condition. The aim of this paper is to present the methods of DRA treatment based on the results of recent studies.
Topics: Abdominoplasty; Conservative Treatment; Exercise Therapy; Female; Humans; Pregnancy; Pregnancy Complications; Prolotherapy; Prune Belly Syndrome
PubMed: 29512814
DOI: 10.5603/GP.a2018.0016 -
Fetal and Pediatric Pathology 2014This article reviews the majority of Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) with emphasis in Pediatric Pathology describing and illustrating... (Review)
Review
This article reviews the majority of Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) with emphasis in Pediatric Pathology describing and illustrating lesions as varied as ureteral duplications, ureteropelvic junction obstruction, horseshoe kidney, posterior urethral valve and prune belly syndrome, obstructive renal dysplasia, nonmotile ciliopathies and several syndromes associated with renal malformations (Meckel-Joubert, short rib, Bardet-Biedl, asplenia/polysplenia, hereditary renal adysplasia, Zellweger, trisomies, VACTER-L, Potter, caudal dysplasia, and sirenomelia), as well as ADPK, and ARPK. The purpose of this review is not only to describe the congenital renal anomalies, but also to analyze the more recent therapeutic interventions that may modify the natural history of some of these severe conditions.
Topics: Child; Humans; Kidney; Urogenital Abnormalities
PubMed: 25313840
DOI: 10.3109/15513815.2014.959678 -
The Urologic Clinics of North America Nov 2018The field of transitional urology has taken on an increasing importance in recent years as more individuals with congenital urologic issues are living and thriving into... (Review)
Review
The field of transitional urology has taken on an increasing importance in recent years as more individuals with congenital urologic issues are living and thriving into adulthood. This article reviews the transitional process itself including barriers to successful transition and the consequences of failing to properly transition. Also provided is a broad overview of the urologic issues faced by patients who may benefit from lifelong care and the providers who will be helping them with transition and assuming their care.
Topics: Humans; Pediatrics; Transition to Adult Care; Urologic Diseases; Urology
PubMed: 30316314
DOI: 10.1016/j.ucl.2018.06.007 -
Indian Dermatology Online Journal 2022
PubMed: 36225999
DOI: 10.4103/idoj.IDOJ_656_20 -
Advances in Pediatrics Aug 2023Lower urinary tract obstruction (LUTO) is a rare birth defect with a prevalence between 1 in 5,000 and 1 in 25,000 pregnancies. LUTO is one of the most common causes of... (Review)
Review
Lower urinary tract obstruction (LUTO) is a rare birth defect with a prevalence between 1 in 5,000 and 1 in 25,000 pregnancies. LUTO is one of the most common causes of congenital abnormalities of the renal tract. Several genetic conditions have been associated with LUTO. Most common causes of LUTO are posterior urethral valves and urethral atresia. Despite available prenatal and postnatal treatments, LUTO is a significant cause of morbidity and mortality in newborns causing significant end stage renal disease and pulmonary hypoplasia.
Topics: Pregnancy; Female; Humans; Infant, Newborn; Retrospective Studies; Ultrasonography, Prenatal; Urethral Obstruction; Kidney; Urinary Tract
PubMed: 37422291
DOI: 10.1016/j.yapd.2023.03.001 -
Medicina Clinica Dec 2020
Topics: Humans; Prune Belly Syndrome
PubMed: 31780211
DOI: 10.1016/j.medcli.2019.09.002 -
The Surgical Clinics of North America Oct 2022Congenital abdominal wall defects vary from abdominal wall hernias to severe congenital structural anomalies that include gastroschisis, omphalocele, and prune belly... (Review)
Review
Congenital abdominal wall defects vary from abdominal wall hernias to severe congenital structural anomalies that include gastroschisis, omphalocele, and prune belly syndrome. The conditions often carry various associated anomalies and require multidisciplinary treatment approaches. Complex surgical reconstructive techniques are frequently required and prenatal, perioperative, and long-term follow-up is critical to ensuring the best possible outcomes.
Topics: Abdominal Wall; Digestive System Abnormalities; Female; Gastroschisis; Hernia, Umbilical; Humans; Pregnancy; Plastic Surgery Procedures
PubMed: 36209747
DOI: 10.1016/j.suc.2022.07.011